MCID: LMB053
MIFTS: 23

Limbic Encephalitis with Lgi1 Antibodies

Categories: Immune diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Limbic Encephalitis with Lgi1 Antibodies

MalaCards integrated aliases for Limbic Encephalitis with Lgi1 Antibodies:

Name: Limbic Encephalitis with Lgi1 Antibodies 20 58
Limbic Encephalitis with Leucine-Rich Glioma-Inactivated 1 Antibodies 20 58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

ICD10 via Orphanet 33 G04.8
Orphanet 58 ORPHA163908

Summaries for Limbic Encephalitis with Lgi1 Antibodies

GARD : 20 Limbic encephalitis with LGI1 antibodies is a relatively new disease characterized by progressive memory loss, confusion, sleep disturbances, and problems with behaviors and spatial orientation. Seizures are common and frequently precede other symptoms. About half of people with this diagnosis exhibit a specific type of seizure called faciobrachial dystonic seizures, which resemble brief jerks of the face arm. Hyponatremia (low levels of sodium in the blood) may also be present. The disease occurs when antibodies mistakenly attack LGI1 (leucine-rich glioma inactivated 1), a protein found in the brain that binds together two epilepsy -related proteins called ADAM22 and ADAM23. Some people with limbic encephalitis with LGI1 antibodies (5 to 10%) also have a cancer diagnosis (most commonly thymoma and lung cancer), but most do not. The disease is diagnosed through testing of blood or spinal fluid for LGI1 antibodies. If treatment is started when seizures are first noticed, limbic encephalitis may be prevented. Treatment is intravenous or oral corticosteroids, intravenous immunoglobulin (IVIG), plasmapheresis (a process where antibodies are removed from the blood), or a combination of these. Long-term outcome is mostly favorable, although some people are left with some degree of residual impairment. Relapse occurs in up to one third of patients.

MalaCards based summary : Limbic Encephalitis with Lgi1 Antibodies, also known as limbic encephalitis with leucine-rich glioma-inactivated 1 antibodies, is related to limbic encephalitis and encephalitis. An important gene associated with Limbic Encephalitis with Lgi1 Antibodies is HLA-DQB1 (Major Histocompatibility Complex, Class II, DQ Beta 1), and among its related pathways/superpathways are Immune response NFAT in immune response and Th17 cell differentiation.

Related Diseases for Limbic Encephalitis with Lgi1 Antibodies

Diseases related to Limbic Encephalitis with Lgi1 Antibodies via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 74)
# Related Disease Score Top Affiliating Genes
1 limbic encephalitis 10.4
2 encephalitis 10.4
3 pseudobulbar affect 10.2
4 autoimmune polyglandular syndrome type 3 9.9 HLA-DRB1 HLA-DQB1
5 idiopathic hypersomnia 9.9 HLA-DRB1 HLA-DQB1
6 hypersomnia 9.9 HLA-DRB1 HLA-DQB1
7 narcolepsy 2 9.9 HLA-DRB1 HLA-DQB1
8 narcolepsy 9.9 HLA-DRB1 HLA-DQB1
9 htlv-1 associated myelopathy/tropical spastic paraparesis 9.9 HLA-DRB1 HLA-DQB1
10 latent autoimmune diabetes in adults 9.9 HLA-DRB1 HLA-DQB1
11 occupational asthma 9.9 HLA-DRB1 HLA-DQB1
12 primary adrenal insufficiency 9.9 HLA-DRB1 HLA-DQB1
13 recurrent respiratory papillomatosis 9.9 HLA-DRB1 HLA-DQB1
14 beryllium disease 9.9 HLA-DRB1 HLA-DQB1
15 apple allergy 9.9 HLA-DRB1 HLA-DQB1
16 chronic beryllium disease 9.9 HLA-DRB1 HLA-DQB1
17 metal allergy 9.9 HLA-DRB1 HLA-DQB1
18 autoimmune hepatitis 9.9 HLA-DRB1 HLA-DQB1
19 autoimmune hepatitis type 1 9.9 HLA-DRB1 HLA-DQB1
20 focal epithelial hyperplasia 9.9 HLA-DRB1 HLA-DQB1
21 pediatric multiple sclerosis 9.9 HLA-DRB1 HLA-DQB1
22 podoconiosis 9.9 HLA-DRB1 HLA-DQB1
23 pemphigoid 9.9 HLA-DRB1 HLA-DQB1
24 osteonecrosis of the jaw 9.9 HLA-DRB1 HLA-DQB1
25 pityriasis rosea 9.9 HLA-DRB1 HLA-DQB1
26 narcolepsy 1 9.9 HLA-DRB1 HLA-DQB1
27 oligoarticular juvenile idiopathic arthritis 9.9 HLA-DRB1 HLA-DQB1
28 idiopathic nephrotic syndrome 9.9 HLA-DRB1 HLA-DQB1
29 ocular cicatricial pemphigoid 9.9 HLA-DRB1 HLA-DQB1
30 cicatricial pemphigoid 9.9 HLA-DRB1 HLA-DQB1
31 alveolar echinococcosis 9.9 HLA-DRB1 HLA-DQB1
32 paraneoplastic pemphigus 9.9 HLA-DRB1 HLA-DQB1
33 pemphigus 9.9 HLA-DRB1 HLA-DQB1
34 cytomegalovirus retinitis 9.9 HLA-DRB1 HLA-DQB1
35 cystic echinococcosis 9.9 HLA-DRB1 HLA-DQB1
36 pure red-cell aplasia 9.9 HLA-DRB1 HLA-DQB1
37 trachoma 9.9 HLA-DRB1 HLA-DQB1
38 microscopic colitis 9.9 HLA-DRB1 HLA-DQB1
39 autoimmune gastritis 9.9 HLA-DRB1 HLA-DQB1
40 acute disseminated encephalomyelitis 9.9 HLA-DRB1 HLA-DQB1
41 acute chest syndrome 9.9 HLA-DRB1 HLA-DQB1
42 peanut allergy 9.9 HLA-DRB1 HLA-DQB1
43 panuveitis 9.9 HLA-DRB1 HLA-DQB1
44 idiopathic bronchiectasis 9.8 HLA-DRB1 HLA-DQB1
45 guillain-barre syndrome 9.8 HLA-DRB1 HLA-DQB1
46 allergic bronchopulmonary aspergillosis 9.8 HLA-DRB1 HLA-DQB1
47 rheumatic fever 9.8 HLA-DRB1 HLA-DQB1
48 rheumatic heart disease 9.8 HLA-DRB1 HLA-DQB1
49 vogt-koyanagi-harada disease 9.8 HLA-DRB1 HLA-DQB1
50 graft-versus-host disease 9.8 HLA-DRB1 HLA-DQB1

Graphical network of the top 20 diseases related to Limbic Encephalitis with Lgi1 Antibodies:



Diseases related to Limbic Encephalitis with Lgi1 Antibodies

Symptoms & Phenotypes for Limbic Encephalitis with Lgi1 Antibodies

Drugs & Therapeutics for Limbic Encephalitis with Lgi1 Antibodies

Search Clinical Trials , NIH Clinical Center for Limbic Encephalitis with Lgi1 Antibodies

Genetic Tests for Limbic Encephalitis with Lgi1 Antibodies

Anatomical Context for Limbic Encephalitis with Lgi1 Antibodies

Publications for Limbic Encephalitis with Lgi1 Antibodies

Articles related to Limbic Encephalitis with Lgi1 Antibodies:

# Title Authors PMID Year
1
Seizure semiology of anti-LGI1 antibody encephalitis. 20
29171405 2017
2
Anti-LGI1 encephalitis: Clinical syndrome and long-term follow-up. 20
27590293 2016
3
The Treatment and Follow-Up of Anti-LGI1 Limbic Encephalitis. 20
26694143 2016
4
Limbic encephalitis with LGI1 antibodies in a 14-year-old boy. 61
28919330 2018
5
Selective Limbic Blood-Brain Barrier Breakdown in a Feline Model of Limbic Encephalitis with LGI1 Antibodies. 61
29093718 2017
6
A rare case of limbic encephalitis with anti leucine-rich glioma inactivated-1 (LGI1) antibodies. 61
24878970 2014
7
Surgical control of limbic encephalitis associated with LGI1 antibodies. 61
22940785 2012

Variations for Limbic Encephalitis with Lgi1 Antibodies

Expression for Limbic Encephalitis with Lgi1 Antibodies

Search GEO for disease gene expression data for Limbic Encephalitis with Lgi1 Antibodies.

Pathways for Limbic Encephalitis with Lgi1 Antibodies

Pathways related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

(show all 19)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.14 HLA-DRB1 HLA-DQB1
2
Show member pathways
12.11 HLA-DRB1 HLA-DQB1
3
Show member pathways
12.06 HLA-DRB1 HLA-DQB1
4
Show member pathways
11.94 HLA-DRB1 HLA-DQB1
5
Show member pathways
11.9 HLA-DRB1 HLA-DQB1
6 11.88 HLA-DRB1 HLA-DQB1
7
Show member pathways
11.8 HLA-DRB1 HLA-DQB1
8 11.77 HLA-DRB1 HLA-DQB1
9
Show member pathways
11.73 HLA-DRB1 HLA-DQB1
10 11.66 HLA-DRB1 HLA-DQB1
11 11.61 HLA-DRB1 HLA-DQB1
12 11.44 HLA-DRB1 HLA-DQB1
13
Show member pathways
11.44 HLA-DRB1 HLA-DQB1
14 11.38 HLA-DRB1 HLA-DQB1
15 11.32 HLA-DRB1 HLA-DQB1
16 11.24 HLA-DRB1 HLA-DQB1
17 11.12 HLA-DRB1 HLA-DQB1
18 10.81 HLA-DRB1 HLA-DQB1
19
Show member pathways
10.58 HLA-DRB1 HLA-DQB1

GO Terms for Limbic Encephalitis with Lgi1 Antibodies

Cellular components related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 lysosome GO:0005764 9.48 HLA-DRB1 HLA-DQB1
2 lysosomal membrane GO:0005765 9.46 HLA-DRB1 HLA-DQB1
3 endosome membrane GO:0010008 9.43 HLA-DRB1 HLA-DQB1
4 trans-Golgi network membrane GO:0032588 9.4 HLA-DRB1 HLA-DQB1
5 endocytic vesicle membrane GO:0030666 9.37 HLA-DRB1 HLA-DQB1
6 ER to Golgi transport vesicle membrane GO:0012507 9.32 HLA-DRB1 HLA-DQB1
7 transport vesicle membrane GO:0030658 9.26 HLA-DRB1 HLA-DQB1
8 clathrin-coated endocytic vesicle membrane GO:0030669 9.16 HLA-DRB1 HLA-DQB1
9 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.96 HLA-DRB1 HLA-DQB1
10 MHC class II protein complex GO:0042613 8.62 HLA-DRB1 HLA-DQB1

Biological processes related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 adaptive immune response GO:0002250 9.43 HLA-DRB1 HLA-DQB1
2 T cell receptor signaling pathway GO:0050852 9.4 HLA-DRB1 HLA-DQB1
3 antigen processing and presentation of exogenous peptide antigen via MHC class II GO:0019886 9.37 HLA-DRB1 HLA-DQB1
4 interferon-gamma-mediated signaling pathway GO:0060333 9.32 HLA-DRB1 HLA-DQB1
5 antigen processing and presentation GO:0019882 9.26 HLA-DRB1 HLA-DQB1
6 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.16 HLA-DRB1 HLA-DQB1
7 humoral immune response mediated by circulating immunoglobulin GO:0002455 8.96 HLA-DRB1 HLA-DQB1
8 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 8.62 HLA-DRB1 HLA-DQB1

Molecular functions related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 peptide antigen binding GO:0042605 8.96 HLA-DRB1 HLA-DQB1
2 MHC class II receptor activity GO:0032395 8.62 HLA-DRB1 HLA-DQB1

Sources for Limbic Encephalitis with Lgi1 Antibodies

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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